This article titled “The pulmonary arterial hypertension patient perspective”, explores the experiences of patients living with pulmonary arterial hypertension associated with Congenital Heart Disease (PAH-CHD). Thanks to medical advances, many individuals with congenital heart disease are now living longer, but face numerous challenges including physical limitations, mental health issues, relationship strains, financial burdens, and family planning difficulties.
To better understand the patient perspective, the authors interviewed two individuals—Max and Olivia—both in their late 30s and living with pulmonary arterial hypertension associated with congenital heart disease caused by transposition of the great arteries. Despite early diagnosis and lifelong specialist care, they still face significant daily burdens due to their condition. Both are on advanced triple oral therapy.
Key Challenges
- Diagnosis delays: 51% wait over a year; often misdiagnosed
- Physical limitations: Fatigue and breathlessness affect daily activities
- Social impact: 78% report relationship problems; “invisible illness” stigma
- Family planning: Pregnancy carries 11-25% maternal mortality risk
- Mental health: Depression (35-55%) and anxiety common
- Financial burden: 76% experience income reduction; benefit claims difficult
Impact on Daily Life
- Patients adapt activities (Max played goalkeeper, Olivia focused on arts)
- Treatment side effects can worsen quality of life despite clinical improvement
- Strong family support essential but can become overprotective
- Partners provide crucial support but struggle with uncertainty
Support Systems
- Patient organizations (like PHA-UK) provide advocacy and peer support
- Multidisciplinary healthcare approach needed
- Early palliative care recommended alongside active treatment
- Quality of life measures now standard in care
Conclusion
Despite medical advances improving survival, pulmonary arterial hypertension associated with congenital heart disease patients face complex challenges beyond physical symptoms. Effective care requires comprehensive support addressing relationships, mental health, finances, and individual patient goals—not just clinical metrics.
Read full article at this link on the International Journal of Cardiology Congenital Heart Disease
Citation
Daniel A. Jeffery, Wendy Gin-Sing, Radwa Bedair, Luke S. Howard, The PAH patient’s perspective, International Journal of Cardiology Congenital Heart Disease,
Volume 21, 2025, 100596, ISSN 2666-6685, https://doi.org/10.1016/j.ijcchd.2025.100596.
(https://www.sciencedirect.com/science/article/pii/S2666668525000321)
